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Biomedical subjects

B H Bernstein

Publications and source records attributed to B H Bernstein.

At least 19 recordsLinked to original sources
PubMed1997

Preliminary evidence for cyclosporin A as an alternative in the treatment of recalcitrant juvenile rheumatoid arthritis and juvenile dermatomyositis.

OBJECTIVE: To evaluate the safety and efficacy of cyclosporin A (CyA) with and without methotrexate (MTX) in refractory juvenile rheumatoid arthritis (JRA) and juvenile dermatomyositis (JDMS). METHODS: Twenty-two patients (17 with JRA, 5 with JDMS) with refractory disease were studied retrospectively. All received CyA at a mean dose of 3.2 mg/kg/day over a mean period of 16 mo (range 6-42). All other medications except nonsteroidal antiinflammatory drugs, prednisone, and hydroxychloroquine were discontinued. In addition, 16/22 patients received concomitant MTX. RESULTS: Improvements in laboratory variables, joint counts, joint swelling, and morning stiffness were observed in most of the children with JRA. Muscle strength increased and muscle enzyme levels decreased in the patients with JDMS. CyA treatment permitted prednisone to be discontinued in 5/20 and reduced by greater than 50% in 10/20 patients. There was no evidence of hepatic or bone marrow toxicity or lymphoproliferative disease. Serum creatinine increased in 13/22 patients, but the actual values all remained within normal limits. CONCLUSION: CyA may be an effective agent in the treatment of refractory JRA and JDMS and concomitant MTX seems to be well tolerated. These preliminary data also suggest that combined CyA/MTX therapy may be associated with further improvement in clinical outcome.

Adolescent↗
PubMed1996

Effects of perceived stress on pediatric chronic pain.

The dearth of theoretically driven research on the predictors of pediatric chronic pain may unwittingly contribute to needless suffering in children and adolescents by underinvestigating a potentially treatable condition. The objective of the present study was to investigate the hypothesized predictive effects of perceived stress on pediatric chronic pain intensity in 148 children and adolescents. Consistent with the a priori Biobehavioral Model of Pediatric Pain, higher perceived stress was predictive of greater pediatric pain intensity. The results are discussed with regard to the implications for cognitive-behavioral pediatric pain treatment.

Adolescent↗
PubMed1996

Assessing chronic musculoskeletal pain associated with rheumatic disease: further validation of the pediatric pain questionnaire.

Evaluated the validity, stability, and clinical utility of the Pediatric Pain Questionnaire (PPQ), a comprehensive, multidimensional instrument for assessing childhood pain. Previous studies demonstrated adequate psychometric properties of the PPQ using small samples. Results of the current study, using a large sample (N = 100) of children and adolescents with chronic pain associated with rheumatic disease, were consistent with initial validation studies. High correlations were found among visual analog scale (VAS) pain intensity ratings obtained from parents, physicians, and patients. Significant correlations also were found between pain ratings and measures of disease activity and functional status. VAS ratings evidenced moderate stability over a 6-month period in this sample of youth with relatively stable disease activity. Other components of the PPQ provide comprehensive information that is clinically useful for treatment planning and evaluation.

Activities of Daily Living↗
PubMed1996

Chronic pain and emotional distress in children and adolescents.

Pediatric chronic pain continues to be relatively underinvestigated and undertreated. The objective of the present cross-sectional study was to investigate the emotional distress hypothesized to be concurrently associated with the chronic pain experience in children and adolescents. One hundred and sixty children and adolescents with chronic pain and their parents completed standardized assessment instruments measuring pain intensity, depressive symptoms, state anxiety, trait anxiety, general self-esteem, and internalizing and externalizing behavior problems. Consistent with the a priori Biobehavioral Model of Pediatric Pain, higher patient-perceived pain intensity was associated with higher depressive and anxious symptoms, lower general self-esteem, and higher behavior problems. The results are discussed in regard to preventing and treating pain and suffering in children and adolescents with chronic pain.

Adaptation, Psychological↗
PubMed1991

Evaluation and management of pain in children with rheumatic diseases.

The evaluation and management of pain in children with rheumatic diseases are still in the early stages of empirical development. Nevertheless, with the advent of the PPQ, systematic research efforts are now underway to develop the reliability and validity of the PPQ's measurement characteristics for pediatric rheumatic diseases. With these developments, the inclusion of pediatric pain measurement as an essential outcome variable in controlled clinical trials should be advocated. The major points to be advocated by professionals who care for these children are (1) Children can accurately report their pain when age-appropriate measures are used; (2) pharmacologic treatment is not a sufficient condition for adequate pain management; (3) cognitive-behavior therapy techniques can facilitate children's coping with "breakthrough" recurrent musculoskeletal pain with no known side effects; and (4) adequate pain control should be viewed as a quality assurance issue, considered as another indicator of the adequacy of pediatric health care.

Arthritis, Juvenile↗
PubMed1991

Auranofin therapy for juvenile rheumatoid arthritis: results of the five-year open label extension trial.

Eighty-eight children with juvenile rheumatoid arthritis (JRA) who completed a double blind, randomized placebo controlled trial of oral gold were entered into an open label extension phase during which they received auranofin (AF) at a dosage of 0.15-0.2 mg/kg/day (9 mg/day maximum). Eleven (12.5%) patients completed 5 years of AF therapy; 77 (87.5%) did not. Fifteen (17%) of the 88 were in disease remission at the final visit. Mean duration of therapy for those who discontinued was 646 days. Parental/patient decision and insufficient therapeutic effect were the 2 most frequent reasons for early termination, followed by adverse effects. Though relatively well tolerated, AF provides adequate longterm management for only a small percentage of patients with JRA.

Administration, Oral↗
PubMed1989

Systemic lupus erythematosus in the first decade of life.

To evaluate whether the onset of systemic lupus erythematosus in the first decade of life was associated with a unique pattern of racial preponderance, sexual preponderance, genetic predisposition, or disease expression, the medical records of 23 children with systemic lupus erythematosus prior to their tenth birthdays were compared with the medical records of 82 children in whom lupus was diagnosed between their tenth and 20th birthdays. No statistically significant differences in sex distribution, racial (ethnic) background, family history, mode of onset, morbidity, or mortality rates were found between the two age groups. The frequently held view that children with early-onset lupus do worse probably relates to the fact that even though they survive as long as children with the older-onset disease, they die younger because they have the onset of their lupus at a younger age.

Adolescent↗
PubMed1987

The prevalence of coagulation abnormalities in juvenile rheumatoid arthritis.

To determine the prevalence of coagulopathy in juvenile rheumatoid arthritis, results of repeated coagulation studies obtained on 73 patients over one year were correlated with disease activity, liver function abnormalities and drug therapy. In spite of active and severe disease in the majority of these children, coagulation abnormalities developed in only 2 cases and there was no instance of clinically apparent bleeding. Although these results suggest that the development of coagulopathy is uncommon, the physician must continue to exercise vigilance for this potentially life threatening complication, especially when caring for the child with systemic disease receiving combinations of drug therapy.

Adolescent↗
PubMed1984

Course of treated juvenile dermatomyositis.

Sixty-six patients with possible juvenile dermatomyositis (JDMS) were observed at the Children's Hospital of Los Angeles from 1960 to 1982. In patients initially given high doses of corticosteroids followed by low-dose therapy, three different clinical courses had previously been observed: monocyclic, polycyclic, and chronic continuous. We reviewed the records of 32 patients who met study criteria. The course of JDMS was monocyclic in eight children, chronic polycyclic in 10, and chronic continuous in 14. Of these children, 25 are well and not receiving medication; one has mild JDMS, without corticosteroid therapy; four have active JDMS despite corticosteroid therapy (one is severely handicapped); and two have died. Our results support the improved prognosis of JDMS after corticosteroid therapy, but also the great clinical variability of the disease. Understanding of this variability, as reflected in the three disease courses, facilitates physician choice of the optimal treatment with the least drug toxicity for the individual patient, continuing efforts to clarify the disease pathogenesis, and research efforts to improve current treatment programs for the patient with severe JDMS.

Acute Disease↗
PubMed1984

Joint calcification following intra-articular corticosteroid therapy.

The authors conducted a blind retrospective survey of the clinical records and radiographic examinations of 100 patients with juvenile rheumatoid arthritis to ascertain the cause of the joint calcification observed in several cases. Review of the clinical histories showed that 39 patients (92 joints) had been treated with intrasynovial corticosteroids, while an independent study of the radiographs of the same 100 children revealed 20 cases of calcification involving 32 joints. All of the calcified joints had been injected with intrasynovial corticosteroids on one or more occasions. The frequency of calcification, its morphology, and the possible pathogenesis are discussed.

Adolescent↗
PubMed1978

Total hip replacement in children with arthritis.

Fourteen children with juvenile rheumatoid arthritis and two with ankylosing spondylitis received 29 total hip replacements (THR). The ages at THR were 12 to 18 years, the median duration of prior hip disease was 7.1 years, and the minimum followup was one year (range 1--4 years, median 2.2 years). The primary indication for THR was pain in 5 hips and severe malposition or flexion contractures in 24 hips. (Sixteen of these also had some degree of pain.) All 29 hips demonstrated improved postoperative range of motion, and all were free of pain. In children, active rheumatic disease in other joints, prosthesis longevity, and possible lack of adequate motivation all merit special consideration prior to THR, but the results are encouraging.

Adolescent↗
PubMed1978

Reflex neurovascular dystrophy in childhood.

Reflex neurovascular dystrophy has rarely been recognized in children. During the past eight years we have observed 24 instances of RND in 23 children. Lower extremity involvement was manifested in 20 of them and upper extremity in four. The major complaint was pain; swelling and vasomotor instability were prominent, and exquisite tenderness was characteristic. Chronic trophic changes were not observed. Antecedent illness or trauma could be related to the RND in less than half of the children, but personality factors appeared contributory to the development of RND in most children. Physical therapy was the principal form of treatment; therapy with a corticosteroid or by sympathetic blockade was not employed. Reduction in the evidences of disease, including improvement in function, were present in all children at the termination of therapy; improvement was maintained in all but one child after a mean period of 2.4 years. The excellent response to conservative therapy suggests that RND may be a more benign condition in children than in adults.

Adolescent↗
PubMed1977

Aspirin-induced hepatotoxicity and its effect on juvenile rheumatoid arthritis.

Evidence of hepatic disease was sought in 102 children with juvenile rheumatoid arthritis (JRA) who were treated with aspirin. Serum glutamic oxaloacetic transaminase level was elevated (greater than 39 IU/liter) in 59% of the children. The degree and prevalence of SGOT elevations correlated with aspirin dose and serum salicylate level. Nevertheless, increased SGOT values were frequently present in children receiving moderate aspirin doses and having serum salicylate levels less than 25 mg/100 ml. Elevated SGOT values decreased in proportion to the degree of reduction in aspirin dose. The SGOT values above the 100 IU/liter were statistically associated with reduced sedimentation rates. Concomitant improvement in the clinical manifestations of JRA was noted in some children.

Arthritis, Juvenile↗
PubMed1977

Mixed connective tissue disease in childhood. A clinical and serologic survey.

Mixed connective tissue disease is a syndrome with overlapping clinical features of SLE, scleroderma, and polymyositis. Only one other child with MCTD has been described in detail. In this study 14 children with MCTD are described. Each had overlapping clinical findings that evolved over an extended period of observation, and all 14 had high serum titers of speckled ANA and antibodies to RNP. A serologic survey of 127 children with various rheumatic diseases confirmed the specificity of high titer of speckled ANA and antibodies to RNP for MCTD in children. Significant cardiac and renal involvement, and thrombocytopenia, may be more common in affected children than in adults with MCTD, may lead to longer therapy with higher doses of a corticosteroid, and may contribute to a more serious prognosis than in adults.

Adolescent↗
PubMed1977

Minoxidil therapy in children with severe hypertension.

Six children, from 1.3 to 18 years of age, with severe hypertension associated with the hemolytic uremic syndrome, periarteritis, and renal transplant rejection received minoxidil, an antihypertensive agent, for three to 36 weeks. All had severe hypertension resistant to oral antihypertensive medications; five required frequent intravenous diazoxide therapy prior to minoxidil therapy. The mean pretreatment systolic and diastolic blood pressures were 176 and 117 mm Hg, respectively. Following treatment, the mean systolic and diastolic blood pressures were 133 and 82 mm Hg, respectively. Concomitant antihypertensive medications were decreased in all six patients once optimal blood pressure control was obtained. The initial dosage of minoxidil was 0.1 to 0.2 mg/kg/day; maximal dosage for blood pressure was 0.3 to 1.4 mg/kh/day. Major complications of therapy were fluid retention and hirsutism. Transient asymptomatic pericardial effusions occurred in two patients. Three patients on prolonged minoxidil therapy had persistent increases in right ventricular end diastolic diameters. Minoxidil is an effective oral antihypertensive agent for treatment of severe hypertension in pediatric patients. Avoidance of fluid retention is mandatory to prevent congestive heart failure.

Adolescent↗